Truncus arteriosus, also known as common arterial trunk is an unusual embryologic anomaly. It is characterized by incomplete conotuncal septation resulting in a common aortopulmonary trunk and ventricular septal defect. The first surgical repair was performed in 1962 when Behdtrendt et al. closed the VSD and used a valveless conduit to establish right ventricle-pulmonary artery continuity. Complete repair of this anomaly with a valved conduit was first reported in 1967 by McGoon et al. and remains the procedure of choice for these patients. National Cardiothoracic Center of Excellence was established in 1984. Since then it had carried out significant surgical management of simple CHD. Recently, with the aid of foreign medical missions, it was able to carry out a successful repair of truncus arteriosus type 1, the first of its kind in Nigeria.